Pulmonology

Wheezing and Asthma

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Wheezing

  • Definition: Continuous high-pitched sound with musical quality emitting from the chest during expiration.
  • Patterns of wheezing (J Allergy Clin Immunol 2003;111(4):661): According to the Tucson children’s respiratory study, a child who begins wheezing in the early years of life (<3 yr) can be classified into one of the following three phenotypes:
    1. Transient early wheezing (not associated with eczema, other atopy, or family hx of asthma)

      • Comprises 80% of children who wheeze during the first year of life and 60% of children during second year of life.
      • Wheezing episodes resolve by age 3 yr.
      • Risk factors include low infant pulmonary function, maternal smoking during pregnancy, younger mother.

    2. Non-atopic wheezing

      • Children who continue to wheeze beyond the third year of life after having an RSV or other viral LRI early in life.
      • RSV-LRI predisposes to lower lung function and more likely bronchodilator response.

    3. IgE-associated or atopic wheeze or asthma

      • Episodes more likely to continue beyond age 6 yr.
      • Associated with markers of atopy (high IgE, eosinophilia).
      • Family hx of asthma (especially in the mother), increased other atopy (eczema, rhinitis).
      • Lowest lung function at age 6 yr compared with other phenotypes.

  • Differential diagnosis: Modified asthma predictive index (API; J Allergy Clin Immunol 2004;114(6):1282): Identifies children with wheezing who are at risk for persistent asthma; requires h/o four or more episodes of childhood wheezing and one major and two minor criteria
  • See table below

Differential Diagnosis for a Child with Wheezing

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Age <5 Years

Age >5 Years

  • Asthma
  • Infection (viral upper or lower respiratory infections, bronchiolitis, tuberculosis, pertussis)
  • Bronchopulmonary dysplasia
  • Sinusitis
  • Foreign body aspiration
  • Anatomic abnormality (vascular ring, mediastinal mass)
  • Tracheobronchomalacia
  • Aspiration due to swallow dysfunction or GERD
  • Cardiac disease with congestive heart failure
  • Immunodeficiency, immotile cilia
  • CF
  • Asthma
  • Vocal cord dysfunction
  • GERD
  • CF

Modified Asthma Predictive Index

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Major Criteria

Minor Criteria

Parental history of asthma

Physician-diagnosed atopic dermatitis

Allergic sensitization to at least one aeroallergen

Allergic sensitization to eggs, milk, or peanuts

Wheezing apart from viral illness

Blood eosinophilia >4%

Asthma

  • Definition: Chronic inflammatory disorder of the airways associated with airway hyperresponsiveness that leads to recurrent episodes of combinations of wheezing, breathlessness, chest tightness, or coughing. Usually associated with diffuse but variable airflow obstruction within the lung that is often reversible spontaneously or with treatment.
  • Pathophysiology: Combination of bronchial smooth muscle constriction and obstruction of the lumen (by inflammatory exudates and airway wall edema).

Diagnosis

Clinical History and Physical Exam for Diagnosing Asthma

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History

Physical Exam

  • Age at onset of wheezing
  • Frequency, timing (day or night)
  • H/o prematurity, BPD
  • Use of urgent care ED visits, hospitalizations, need for O2 or intubation
  • Comorbid conditions: Eczema, allergies, chronic rhinitis, sinusitis, GERD
  • FHx of asthma and allergic conditions
  • Triggers for wheezing: URI or infection, exercise, cold air, allergens (eg, dust mite, animal dander, grass or tree pollen, molds, cockroach), irritant exposures (eg, tobacco smoke, air pollution), emotional stress
  • Environmental exposures: Pets, tobacco smoke, carpet, dust, cockroaches, central air or heat, use of pillow or mattress covers (dust mite exposure)
  • Positive response to short-acting bronchodilator
  • Current medications (check adherence and drug delivery technique)

  • Respiratory: Evaluate degree of air entry; evaluate for prolonged expiratory phase, tachypnea, retractions (subcostal, sternal, intercostal), wheezing vs stridor
  • Signs of atopy: Eczema, rhinorrhea, transverse nasal crease (allergic salute), Dennie’s lines (folds inferior to the lower eyelid), allergic shiners (periorbital darkening), conjunctivitis, pale or edematous nasal turbinates
  • Absence of clubbing
  • Normal growth
  • Diagnostic studies
    • CXR: May be normal or may show hyperinflation (flattened diaphragm chest A/P diameter) or peribronchial thickening.
    • Peak flow monitors: May be useful in children ≥6 yr, especially those with poor symptom perception. Used to assess the severity of exacerbations, monitor response to therapy (compare with personal best at baseline or can estimate goal PEF using predicted table by gender, height, age). Limitations include that it is effort dependent and requires training (can have false high and low values).
    • Spirometry: Obstructive pattern with ↓ FEV1 and ↓ FEV1/FVC ratio; scooped or concave pattern on expiratory flow–volume loop.
    • Positive bronchodilator (BD) response = ↑ of ≥12% in FEV1 after inhaled short-acting □-agonist.
    • Bronchial provocation tests: May use exercise, histamine, or methacholine to provoke airway hyperresponsiveness with recovery post-BD.
  • Management: There are four components to asthma management (NAEPP EPR-3, 2007, http://www.nhlbi.nih.gov.easyaccess2.lib.cuhk.edu.hk/guidelines/asthma):
    • Component 1: Asthma assessment (see table below) and objective monitoring (see table “Assessing control and adjusting therapy”)
    • Component 2: Education for a partnership in asthma care
    • Train families in self-management skills, including monitoring, treatment, and communication.
    • Define goals for good asthma control. Goals should include (1) control symptoms, allowing normal levels of activity and undisturbed sleep; (2) prevent exacerbations; (3) maintain normal lung function; and (4) use minimal therapy necessary to minimize side effects.
    • Provide a written asthma action (management) plan and emergency information.
      • Printable asthma action plan template: NHLBI EPR3, page 402 (http://www.nhlbi.nih.gov.easyaccess2.lib.cuhk.edu.hk/guidelines/asthma/asthgdln.pdf)
      • Printable MDI instructions: NHLBI EPR3, page 403(http://www.nhlbi.nih.gov.easyaccess2.lib.cuhk.edu.hk/guidelines/asthma/asthgdln.pdf)
    • Component 3: Control of environmental factors and comorbid conditions that affect asthma
    • Define specific allergy sensitization (skin testing or RAST/ImmunoCap) and avoid exposure; consider immunotherapy.
    • Eliminate exposure to active or passive tobacco smoke; limit exposure to air pollutants.
    • Manage comorbid conditions (GERD, allergic rhinitis).
    • Annual influenza vaccine, good handwashing.
    • Component 4: Pharmacologic therapy
    • All patients need access to quick-relief bronchodilator (short-acting β2-agonist).
    • Daily controller therapy based on severity and control; consider both impairment and risk (See tables “Medications commonly used for treating Asthma” and “Stepwise approach to management of Asthma” below).
    • Inhaled drug delivery technique is important to avoid frequent errors; check regularly.
    • MDI with a spacer has similar or better efficacy, more portability and a shorter administration time compared to a nebulizer.
    • Spacer (valved holding chamber) with MDI improves lower airway delivery at all ages (including adults) and reduces side effects (thrush with ICS).

Classifying Asthma Severity and Initiating Therapy*

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Components of severity

Intermittent

Persistent

Mild

Moderate

Severe

Impairment

Symptoms

≤ 2 days/wk

>2 days/ wk

Daily

Throughout the day

Nighttime awakenings

0-4 yr: 0

≥5 yr: ≤ 2x/mo

0-4 yr: 1-2x/mo

≥5 yr: 3-4x/mo

0-4 yr: 3-4x/mo

≥5 yr: >1x/wk

0-4: >1x/wk

≥5 yr: Often, 7x/wk

SABA use for symptoms

≤2 days/wk

>2 days/wk

Daily

Several times per day

Interference with normal activity

None

Minor

Some

Extreme

Lung function

Normal FEV1 between exacerbations

FEV1 >80% predicted

5-12 yr: FEV1/FVC >85%

≥12 yr: FEV1/FVC normal

FEV1 >80% predicted

5-12 yr: FEV1/FVC >80%

≥12 yr: FEV1/FVC normal

FEV1: 60%-80% predicted

5-12 yr: FEV1/FVC 75%-80%

≥12 yr: FEV1/FVC reduced 5%

FEV1 < 60% predicted

5-12 yr: FEV1/FVC< 75%

≥12 yr: FEV1/FVC reduced >5%

Risk

Exacerbations requiring oral corticosteroid

0-1/yr

0-4 yr: ≥2 in 6 mo OR ≥4 wheezing episodes per 1 year lasting >1 day

≥5 yr: ≥2/yr

Recommended Initial Therapy

(see table “Stepwise approach to management of asthma” below)

Step 1

Step 2

Step 3

0-4 yr: Step 3

5-12 yr: Step 3 or 4

≥12 yr: Step 4 or 5

and consider short course of oral systemic steroids

In 2-6 weeks, evaluate level of asthma control and adjust therapy accordingly.

*Some criteria vary by age.

Adapted from NHLBI, EPR 3, 2007:41

Assessing Control and Adjusting Therapy*

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Components of Severity

Well Controlled

Not Well Controlled

Very Poorly Controlled

Impairment

Symptoms

≤2 d/wk

>2 d/wk

Throughout the day

Nighttime awakenings

0–11 yr: ≤1x/mo

≥12 yr: ≤2x/mo

0–11 yr: ≥2x/mo

≥12 yr: 1–3x/wk

0–11 yr: ≥2x/wk

≥12 yr: ≥4x/wk

Interference with normal activity

None

Some limitation

Extremely limited

SABA use for symptoms

≤2 days/wk

>2 days/wk

Several times per day

Lung function

FEV1 or peak flow

FEV1/FVC (> = 5 yr)

>80% predicted or personal best

>80%

60%–80% predicted or personal best

75%–80%

<60% predicted or personal best

<75%

Risk

Exacerbations requiring oral corticosteroids

0–1x/yr

<5yr: 2–3x/yr

≥5yr: ≥2x/yr

<5 yr: >3x/yr

≥5 yr: ≥2x/yr

Treatment-related adverse effects

Medication side effects do not correlate with specific levels of control but should be considered in the overall assessment of risk

Recommended Action for Treatment

Maintain current step

Regular follow-up q 1-6 mo

Consider stepdown if well controlled for ≥3 mo

Step up 1 step and reevaluate in 2–6 wk

Consider short course of oral corticosteroid

Step up 1–2 steps and reevaluate in 2 wk

For side effects, consider alternative treatment options

Adapted from NHLBI, EPR 3, 2007:40,43

Medications Commonly Used for Treating Asthma

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Medication Class

Examples

Adverse Effects

Comments

Short-acting β-agonist (SABA)

Albuterol

Levalbuterol

Tachycardia, nervousness, tremors

Short-term symptom relief and pre-exercise

Systemic corticosteroid

Prednisolone

Prednisone

Methylprednisolone

Dexamethasone

Altered behavior, nausea or vomiting, pituitary adrenal axis suppression (long-term therapy)

Short courses are effective for exacerbations or establishing control at initiation of therapy

High-dose or extended therapy should be weaned as tolerated

Inhaled corticosteroid (ICS)

MDI: Fluticasone, beclomethasone, flunisolide, triamcinolone

DPI: Budesonide, mometasone

Nebulized: Budesonide

Thrush

Decreased height velocity* (temporary)

Adrenal suppression (high dose)

Mainstay of preventive therapy

Different ICS have similar efficacy but different potency

High-dose therapy should be weaned

ICS + long-acting β2-agonist (LABAs)

Fluticasone + salmeterol

Budesonide + formoterol

As above for ICS, Headache

FDA alert: LABAs should not be used for symptom relief or without concomitant ICS

Leukotriene receptor antagonist

Montelukast

Zafirlukast

Headache, nausea, cough

FDA alert: Rarely neuropsychiatric events have been reported. Also used for allergic rhinitis

Anti-IgE monoclonal antibody

Omalizumab

Injection site reaction

Anaphylaxis

For use in persistent allergic asthma refractory to high-dose ICS

*For further discussion see NHLBI EPR3 page 222 (http://www.nhlbi.nih.gov.easyaccess2.lib.cuhk.edu.hk/guidelines/asthma/asthgdln.pdf)

Stepwise Approach to Management of Asthma*

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Preferred Treatment

Step 1

SABA PRN

Step 2

Low-dose inhaled corticosteroid (ICS)

Step 3

0–4 yr: Medium-dose ICS

5–11 yr: Low-dose ICS + either LABA, LTRA, or theophylline; OR medium- dose ICS

≥12 yr: Low-dose ICS + LABA; or medium-dose ICS

Step 4

0–4 yr: Medium-dose ICS + either LABA or montelukast

≥5 yr: Medium-dose ICS + LABA

Step 5

0–4 yr: High-dose ICS + either LABA or montelukast

≥5 yr: High-dose ICS + LABA (consider omalizumab for patients ≥12 yr with allergies)

Step 6

0–4 yr: High-dose ICS + either LABA or montelukast + oral systemic steroid

≥5 yr: High-dose ICS + LABA + oral systemic steroid (consider omalizumab for patients ≥12 yr with allergies)

* Refer to NHLBI EPR3 (http://www.nhlbi.nih.gov.easyaccess2.lib.cuhk.edu.hk/guidelines/asthma/asthgdln.pdf) for alternative treatments.

The stepwise approach is meant to assist, not replace, clinical decision making. If clear benefit is not observed within 4–6 wk when patient technique and adherence is satisfactory, consider adjusting therapy or consider alternative diagnoses. Before stepping up, review adherence, inhaler technique, environmental control, and comorbid conditions.

Consult with asthma specialist if Step 3 care or higher is required.

Adapted from NHLBI, EPR-3, 2007:305, 306.

Cystic Fibrosis

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  • Epidemiology: 30,000 patients in the US; incidence of CF in US by racial or ethnic group: Caucasians, one in 2500; Hispanics, one in 8500; African Americans, one in 15,000; Asian Americans, one in 31,000.
  • Genetics
    • AR transmission; defect in a single gene on chromosome 7 that encodes a chloride channel called the cystic fibrosis transmembrane regulator (CFTR).
    • The ΔF508 mutation is the most common mutation, present in ∼70% of CF alleles in the US; however, >1500 CFTR mutations have been identified.
  • Diagnosis: CF is a clinical diagnosis that requires laboratory corroboration.

Criteria for the Diagnosis of Cystic Fibrosis

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  • ≥1 characteristic phenotypic feature or
  • Positive family history of CF or
  • Positive CF newborn screen result

Plus

Evidence of abnormal CFTR function as demonstrated by one of the following:

  • Elevated sweat chloride ≥60 mmol/L on ≥2 occasions*
  • Identification of two disease-causing CFTR gene mutations
  • Abnormal nasal transepithelial ion transport

*Should be performed at CF center or experienced laboratory.

Adapted from J Pediatr 1998;132(4):589.

  • Sweat chloride test: Pilocarpine iontophoresis is the gold standard for CF diagnosis but is not always conclusive (see table “Reference values for sweat chloride test” below). Sweat test can be done starting at age 2 weeks.
  • Sequencing of all known CFTR DNA mutations can identify 90% of CF mutations.
  • Sweat chloride levels and genotype analysis cannot be used to predict prognosis.
  • Newborn screen (NBS) can identify newborns at risk for CF. NBS protocols for screening vary by state, but all NBS identify blood level of immunoreactive trypsinogen (IRT).
    • First NBS ⊕ → Refer to a CF center for repeat testing. Testing is based on your state’s NBS program (IRT testing repeat, sweat test, CF DNA mutation analysis or a combination of these tests).
      • The panel of CF DNA mutations analyzed varies by state, therefore this test can miss rare mutations. If a patient has borderline sweat test and/or clinical suspicion for CF then sequencing of all known CFTR DNA mutations should be performed.

Reference Values for Qualitative Pilocarpine Iontophoresis Sweat Chloride Test*

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Normal (CF Unlikely)

Indeterminate**

Abnormal (CF Likely)

Infants <6 mo

≤29 mmol/L

30–59 mmol/L

≥60 mmol/L

≥6 mo

≤39 mmol/L

40–59 mmol/L

≥60 mmol/L

*Sweat test can be performed starting at 2 weeks of age.

** Sweat chloride values in indeterminate range and clinical concern → full sequence CFTR DNA sequencing to establish the diagnosis.

Common Symptoms and Signs of Cystic Fibrosis in Various Age Groups

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Infancy

Childhood

Adolescence and Adulthood

Meconium ileus

Obstructive jaundice

Failure to thrive

Hyponatremic dehydration or metabolic alkalosis

Edema with hypoproteinemia and anemia

Rectal prolapse

Recurrent pneumonia or bronchiolitis

Salty-tasting skin

Acute salt depletion

Chronic metabolic alkalosis

Failure to thrive

Steatorrhea

Distal intestinal obstruction

Chronic sinopulmonary disease

Recurrent pneumonia

Chronic cough and sputum

“Atypical” asthma with bronchiectasis, clubbing

Persistent CXR abnormalities

Pansinusitis

Nasal polyps

Symptoms of vitamin A, D, E, or K deficiency

Heat prostration with hypoelectrolytemia

Delayed sexual development

Obstructive azoospermia or infertility

Congenital absence of the vas deferens

Chronic bronchitis with Pseudomonas aeruginosa

Bronchiectasis or clubbing

Pansinusitis

Chronic abdominal pain

Idiopathic pancreatitis

Cirrhosis

Distal intestinal obstruction

Diabetes mellitus

Hemoptysis

Pneumothorax

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Jan 9, 2019 | Posted by in PEDIATRICS | Comments Off on Pulmonology

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